For Those With Spina Bifida, Medical Advances Lead To Longer Lives
The voices of the characters from “Toy Story” carried through the waiting room as Tammy Williams waited for her clinic appointment at Nationwide Children’s Hospital in Columbus, Ohio on a recent Friday morning.
You might figure that a 46-year-old would prefer something else, but Williams said she doesn’t mind the Disney Channel.
And it suits most of the patients, after all. Williams, as the oldest patient in the hospital’s spina bifida clinic, is used to being surrounded by kids and parents when she sees her team of caregivers at her annual visit.
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Williams is modest and matter-of-fact, quick to mention that she has been more fortunate than some others born with the disfiguring condition. She lost her best friend to spina bifida and sees others who struggle more with managing its complications.
But good news is becoming more abundant. A 10-year-old across the waiting room at Children’s can look at Williams and see promise and hope. She can see independence and a full life that could include college, a career, marriage and children.
The past few decades have been transformative for people born with spina bifida. For the first time, caregivers are seeing patients in their 40s and 50s, said Vickie Hobensack, a nurse practitioner who has worked at Children’s for more than three decades and in the clinic for 22 years.
Williams is in good health and is getting to the point, she said, “where I’m probably more concerned about aging things than the spina bifida.”
She has been seen at Children’s since she was 5 years old, save for a stretch out of state during her first marriage.
Now, she’s married to Scott and has a 17-year-old daughter, whom they adopted, and a grown stepson. The family lives in Mechanicsburg, and Williams works in billing at Children’s, where she first served as a candy striper as a teen.
“It’s kind of cool when you grow up in the clinic,” she said before her annual appointment, which stretched for about four hours and included visits by at least eight caregivers.
The hospital hosts the clinic on Fridays to ease care of those with spina bifida. There, they see a host of experts back-to-back rather than having to make multiple appointments at different offices.
Spina bifida, which translates to “split spine,” is the most common permanently disabling birth defect in the United States. Each day, about eight babies are born with the disorder or a similar defect of the brain and spine, according to the Spina Bifida Association.
The cause of the condition is unclear, but scientists say it’s likely a combination of genetic and environmental factors, including folic-acid deficiency in moms.
Williams’ doctors diagnosed the most severe form, myelomeningocele, though she was born with a thin covering over her birth defect. Most babies in this category have an opening in the back, exposing parts of the spinal cord and nerves.
Most of these children have extra fluid on their brains and might have brain damage. Williams does not.
Other challenges linked to the nerve damage associated with severe spina bifida include mobility problems and trouble going to the bathroom. The degree of disability is more severe the higher up the spine the birth defect is located. Williams’ is in her lower back.
The Spina Bifida Association says that in today’s medical and social environments, about 90 percent of children live to be adults. About 80 percent have no intellectual disabilities. The most significant medical advances that have given children greater hope for full lives include improved shunting techniques that divert fluid from the skull, and urinary catheterization techniques that reduce kidney damage and decrease the chance of kidney failure, Hobensack said.
Careful skin care, including monitoring for pressure sores, also is essential to avoiding infections and sepsis, which can be deadly.
Hobensack said she’s seeing patients flourish into their fourth decade for another reason, too.
“It starts with good parenting: teaching kids independence,” she said.
In some cases, cognitive deficits limit people with spina bifida from going to college or working full time, but finding a good fit in vocational school or volunteer opportunities can be important, Hobensack said.
“You’re out there interacting with people. You are participating in society. Everybody feels better when they’re doing something,” she said.
“When I started here, I had families that were told to take their child home and love them, that the expected outcome wasn’t good.”
Williams had surgery in 1969 when she was 19 days old. She started out in a school for children with special needs but was mainstreamed.
She has used crutches since she was a toddler and has driven a car with hand controls since she was 19. She uses a wheelchair only if she has to do a lot of walking — at a fair, for example, or at the spina bifida camp where she serves as a counselor.
“I’m one of the better stories. I don’t have to use a wheelchair to get around. I don’t have a lot of medical issues or social issues.”
In the decades she has seen the team at Children’s, they’ve become friends, even family. They know one another’s lives, cheer successes and comfort one another in trying times. Some of them attended her wedding.
“I kind of knew at a young age that I didn’t want to be living at home the rest of my life,” Williams said. “I was kind of brought up in a situation that you adapt.”
Like Hobensack, Williams said that good mental health and a strong social network can help someone with spina bifida thrive. “It’s not my life. It’s just a part of my life.”
During her annual appointment, an orthotist helped resolve a nagging spot where her brace irritated a toe.
He told her about the struggles of another patient, who is 25.
“Tell her to see me when she’s 40!” Tammy said with a laugh before asking about his father, who also has treated her, and his sister, who recently moved back to Ohio from Florida.
Later, Dr. Rosalind Batley, a specialist in physical medicine, came in with a student trailing her.
“This is Tammy Williams, who grew up here, pretty much,” said Batley, who has known Williams since she was a teen.
Williams said the fact that the clinic team sees older patients benefits the children as well, because what has unfolded in Williams’ life and in the lives of her contemporaries can inform their care of younger patients as they age.
When Hobensack stopped in for Williams’ final assessment of the day, they went over all the basics: medications, mobility, skin health, and urinary and bowel concerns. And they talked about something that usually doesn’t come up in spina bifida appointments.
Although Williams’ retirement is still pretty far off, Hobensack mentioned that before she stops working she should make sure to get a new wheelchair. And she made sure she was getting her colonoscopies.
“Getting old is not for sissies,” Hobensack said with a laugh.
© 2015 The Columbus Dispatch
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